ATL1 / SPG3A / Atlastin-1 Protein (GST Tag)

Atlastin-1 / Spastic Paraplegia 3 protein A Protein

ATL1 / SPG3A Protein Product Information

• Synonym: ATL1, AD-FSP, FSP1, GBP3, SPG3, SPG3A, atlastin1
• Protein Construction: A DNA sequence encoding the human SPG3A ( NP_056999.2 ) ( Met 1-Thr 447 ) was fused with the GST tag at the N-terminus
• Source: Human
• Expression Host: Baculovirus-Insect cells

ATL1 / SPG3A Protein QC Testing

• Purity: > 80 % as determined by SDS-PAGE
• Endotoxin: < 1.0 EU per μg of the protein as determined by the LAL method
• Stability: Samples are stable for up to twelve months from date of receipt at -70℃
• Predicted N terminal: Met
• Molecular Mass: The recombinant human SPG3A/GST chimera consists of 671 amino acids and and has a calculated molecular mass of 77 KDa. It migrates as an approximately 66 KDa band in SDS-PAGE under reducing conditions
• Formulation: Lyophilized from sterile 50mM Tris, 100mM NaCl, 0.5mM PMSF, 0.5mM EDTA, 0.5mM GSH, pH 8.0
• SDS-PAGE: ATL1 protein

ATL1 / SPG3A Protein Usage Guide

• Storage: Store it under sterile conditions at -70℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.

ATL1 / SPG3A Protein Description

Atlastin-1, also known as Spastic paraplegia 3 protein A, Guanine nucleotide-binding protein 3, GTP-binding protein 3, GBP3, ATL1 and SPG3A, is a multi-pass membrane protein which belongs to the GBP family and atlastin subfamily. ATL1 / SPG3A is expressed predominantly in the adult and fetal central nervous system. Expression of ATL1 / SPG3A in adult brain is at least 50-fold higher than in other tissues. ATL1 / SPG3A is detected predominantly in pyramidal neurons in the cerebral cortex and the hippocampus of the brain. ATL1 / SPG3A is also expressed in upper and lower motor neurons (at protein level). A distinguishing feature of ATL1 / SPG3A is its frequent early onset, raising the possibility that developmental abnormalities may be involved in its pathogenesis. Missense SPG3A mutant atlastin-1 proteins have impaired GTPase activity and may act in a dominant-negative, loss-of-function manner by forming mixed oligomers with wild-type atlastin-1. Defects in ATL1 / SPG3A are the cause of spastic paraplegia autosomal dominant type 3 (SPG3), also known as Strumpell-Lorrain syndrome. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.

References

1. Tessa, A. et al., 2002, Neurology. 59 (12): 2002-5.
2. Zhu, PP. et al., 2006, Hum Mol Genet. 15 (8):1343-53.
3. Meijer, IA. et al., 2007, Ann Neurol. 61 (6): 599-603.
4. Salameh, JS. et al., 2009, J Clin Neuromuscul Dis. 11 (1): 57-9.
5. de Leva, MF. et al., 2010, J Neurol. 257 (3): 328-31.